Aron D C, Findling J W, Fitzgerald P A, Forsham P H, Wilson C B, Tyrrell J B
Endocr Rev. 1982 Summer;3(3):229-44. doi: 10.1210/edrv-3-3-229.
CS comprises a group of disorders characterized by hypercortisolism. The variety of causes--pituitary-dependent CS (CD), adrenal tumor, and the ectopic ACTH syndrome--necessitates a variety of therapies--surgical, radiotherapeutic, and medical. Once a specific diagnosis is made, specific therapy can be instituted. Although some controversy persists regarding treatment, particularly that of CD, for most patients it is straightforward. However, in our experience with more than 60 patients, therapeutic dilemmas can arise in a number of circumstances, e.g. the patient with the radiologically normal sella or recurrent CD after adrenalectomy. In addition, the treatment of such conditions as the large ACTH-producing pituitary tumor, Nelson's syndrome, the malignant ectopic ACTH syndrome, and adrenal carcinoma is not entirely satisfactory. Our approach to these problems is illustrated by seven cases, and we emphasize that the proper management of CS requires both correct diagnosis and the logical application of all available therapies.
库欣综合征(CS)是一组以皮质醇增多为特征的疾病。病因多样——垂体依赖性CS(CD)、肾上腺肿瘤和异位促肾上腺皮质激素(ACTH)综合征——这就需要多种治疗方法——手术、放疗和药物治疗。一旦做出明确诊断,即可开始进行特定治疗。尽管在治疗方面,尤其是CD的治疗方面仍存在一些争议,但对大多数患者来说,治疗方法是明确的。然而,根据我们对60多名患者的经验,在许多情况下可能会出现治疗困境,例如蝶鞍影像学检查正常的患者或肾上腺切除术后复发的CD患者。此外,对于诸如分泌大量ACTH的垂体大肿瘤、尼尔森综合征、恶性异位ACTH综合征和肾上腺癌等疾病的治疗并不完全令人满意。七个病例说明了我们对这些问题的处理方法,我们强调,CS的正确管理需要正确的诊断以及合理应用所有可用的治疗方法。